How do I know if I have Febrile infection-related epilepsy syndrome - FIRES?

Febrile infection-related epilepsy syndrome (FIRES) is a rare, catastrophic form of epilepsy that typically presents as an explosive onset of refractory status epilepticus in a previously healthy child or young adult following a mild febrile illness. Because FIRES is a clinical diagnosis characterized by the sudden development of seizures that do not respond to standard medications, it requires immediate emergency medical evaluation rather than self-assessment.

What are the early warning signs of FIRES?

The hallmark of Febrile infection-related epilepsy syndrome (FIRES) is the sudden onset of frequent, often continuous seizures (status epilepticus) occurring between 24 hours and two weeks after the resolution of a common viral or febrile illness. Unlike typical febrile seizures, which are usually brief and self-limiting, FIRES symptoms involve persistent, drug-resistant seizure activity that requires intensive care management.

When should you seek emergency medical care?

If you or a loved one experiences a prolonged seizure following a recent fever, you must seek emergency care immediately. Febrile infection-related epilepsy syndrome (FIRES) is a medical emergency. You should alert medical staff to the following red flags:

• Seizures that last longer than 5 minutes or occur in rapid succession.


• A change in mental status, confusion, or loss of consciousness following a recent illness.


• Seizures that do not stop despite the administration of rescue medications.


• Development of cognitive or behavioral regression in the weeks following a fever.

How is FIRES diagnosed by specialists?

There is no single blood test for Febrile infection-related epilepsy syndrome (FIRES). Diagnosis is made by a neurologist or epileptologist who rules out other causes of encephalopathy and seizures. Key investigations typically include:

• Continuous video-EEG monitoring to identify the pattern of status epilepticus.


• Lumbar puncture to analyze cerebrospinal fluid for inflammatory markers.


• Comprehensive metabolic and autoimmune panels to rule out genetic or metabolic disorders.


• Advanced neuroimaging, such as high-resolution MRI, to assess for brain inflammation.

How to advocate for yourself or your child?

If you suspect Febrile infection-related epilepsy syndrome (FIRES), clearly communicate the timeline between the initial fever and the onset of seizures. If your concerns are dismissed, request a referral to a tertiary care center or a specialized pediatric epilepsy center. Our community at DiseaseMaps.org currently includes 65 people with Febrile infection-related epilepsy syndrome (FIRES) who understand the importance of early intervention and specialized care.

Next steps

• Consult a board-certified pediatric neurologist or epileptologist immediately.


• Keep a detailed log of the timeline of the initial fever and subsequent seizure activity.


• Connect with the Febrile infection-related epilepsy syndrome (FIRES) community on DiseaseMaps.org to share experiences and resources.


• Request a consultation with a metabolic or genetic specialist if initial testing is inconclusive.

Medical disclaimer: This information is for educational purposes only and does not constitute medical advice; always consult with a qualified healthcare professional regarding clinical diagnosis and treatment.

References

• NIH Genetic and Rare Diseases Information Center (GARD): FIRES.


• Orphanet: Febrile infection-related epilepsy syndrome.


• The NORSE Institute (New-Onset Refractory Status Epilepticus).


• PubMed: Clinical characteristics and outcomes of FIRES (2022 research review).